Mikulicz Resection in Infants and Children: *A 20-Year Survey of 196 Patients

JUDSON G. RANDOLPH, M.D., ROBERT M. ZOLLINGER, JR., M.D.,

ROBERT E. GROSS, M.D.

From the Surgical Service, The Children's Hospital Medical Center, and theDepartment of Surgery, Harvard Medical School, Boston, Massachusetts

IN INFANTS and children many forms ofintestinal obstruction, such as atresia, me-conium ileus, volvulus with gangrene, orother intra-abdominal crisis, require intes-tinal resection. While today, many of thesecan be successfully treated by excision andprimary anastomosis, in some cases, a Mi-kulicz resection with temporary establish-ment of a double-barrelled enterostomycontinues to have certain distinct advan-tages. At the Children's Hospital MedicalCenter in Boston, 196 patients have under-gone a Mikulicz resection in the past 20years. In an effort to sharpen the indica-tions for this surgical maneuver, and toemphasize its value in certain circum-stances, a clinical study of these patientshas been undertaken. Long-term follow upof a large number of these children allowsus to summarize the early and the latecomplications of the Mikulicz operationand to evaluate its role in present-day pedi-atric surgery.

Historical BackgroundSurgeons of the nineteenth century who

attempted colonic or intestinal resectionfound that anastomotic failure and peri-tonitis occurred frequently. To avoid suchcomplications, in 1892, Bloch,3 of Copen-hagen, and Paul,9 of Liverpool, working in-dependently, brought forth a new methodof colon resection. They showed that a

* Presented before the American Surgical Asso-ciation, Phoenix Arizona, April 3-5, 1963.

lesion could be exteriorized and that adouble-barrelled colostomy might be soconstructed that its common wall couldsubsequently be cut down; the resultingsingle stoma could later be closed by aminor extra-peritoneal procedure, thus re-establishing bowel continuity without re-entering the peritoneal cavity. Paul's origi-nal description has an appealing clarity andsoundness of mechanical principle (Fig. 1):

"I therefore thought out and determined toput in practice the following mode of operating inthe next case:-First excise the strictured portionof bowel as in the last two cases; then suture to-gether the cut edges of the mesentery and theadjacent sides of the two ends of the colon, insuch a manner that they would adhere togetherfor about three inches, in the position of the twobarrels of a double-barrelled gun. If this suc-ceeded, the spur might be demolished without theslightest risk of peritonitis, and to such an extentas to insure a free passage and easy closing ofthe artificial anus."

This description remains as a remarkablyaccurate account of the operation as per-formed today.

Since its inception, this form of bowelresection has enjoyed a variegated career,marked by events in the development ofabdominal surgery. At the turn of the cen-tury, it was von Mikulicz,8 of Breslau, whoemphasized and popularized the safetyfactors of this procedure which offered theadvantages of a secure, aseptic, extra-peri-toneal colon reconstruction without thedangers of a leaking anastomosis. This be-

481

482 RANDOLPH, ZOLLINGER AND GROSS

FIG. 1. The first illustration of the double-barrelled enterostomy. (Reproduction from Paul'sarticle in the Liverpool Medico-Chirurgical J

1895.)

came known as the Mikulicz procedureand was widely adopted. In America,Rankin'0 was the chief advocate of theobstructive resection as he termed it, andhe showed its usefulness in certain situa-tions.With great advances in all forms of ab-

dominal and intestinal surgery in the 1920'sand 1930's, primary anastomosis graduallysupplanted the Mikulicz procedure. Fur-thermore, with the advent of more radicalresection for cancer of the colon, theMikulicz operation was clearly shown tobe an inferior treatment for malignancy.It fell not only into disuse, but even intodisrepute.

It is interesting that a comparable de-velopmental history has followed the use

of the Mikulicz resection in intestinal sur-

Annals of SurgerySeptember 1963

gery of infants and children. In the 1930'swhen many children died of intussuscep-tion, Ladd and Gross 7 applied the Mikuliczprinciple to these desperately-ill subjects.The aseptic resection proved to be swift,and allowed immediate isolation of the ne-crotic segment, without prolonging undulythe operation. Subsequently, this form ofresection proved useful for treatment ofmeconium ileus, ileal atresia, and otherintestinal pathology of the newborn infant.With increasing interest in surgical prob-lems of children and infants in the pastdecade, there has been a growing emphasison resection and primary anastomosis ineven the smallest of subjects. 2, 4-6, 13 Suchendeavors have proved safe in many cases,and demonstrate advancement in pediatricsurgical technics. Certain specific condi-tions remain, however, for which the Mi-kulicz double-barrelled enterostomy is thesounder choice of operation.

IndicationsIndications for a Mikulicz resection fall

into four principal categories.I. Meconium Ileus. Infants born with

meconium ileus have two clinical problems.The sticky meconium, deficient in pancre-atic enzymes, forms an immovable masswhich obstructs the lower ileum and colon(Fig. 2A). In addition, all of these childrensuffer from generalized mucoviscidosis,which produces thickened bronchial secre-tions and causes recurrent pulmonary in-fection which can be severe or even fatal.It is essential that such babies have surgi-cal relief of the intestinal obstruction byuse of an operative procedure and choiceof anesthesia which will minimize pulmo-nary complications. Here, the Mikulicz re-section is superior because: the durationof operation is short, the enlarged ob-structed ileal segment is quickly excised,the bowel is immediately decompressedand can be freely irrigated, there is noanastomosis which would have to standthe obstructive stress imposed by any me-

MIKULICZ RESECTION IN INFANTS AND CHILDREN 483NW

L".DMU-_FIG. 2. Some of the indications for a Mikulicz resection. A. Meconium Ileus. B. Intestinal atresia.

(The tiny distal segment has been sutured to the huge proximal segment.) C. Gangrenous intussus-ception. D. Neonatal perforation and necrosis, which required resection.

conium which remains in the collapsedcolon. If the status of the infant is pre-

carious, this operation can be performedunder local anesthesia, minimizing any pul-monary complications.

II. Congenital Intestinal Obstructionwith Disparity in Size of Lumina. In theneonate, intestinal surgery has improvedmarkedly in the past two decades, so thatsome congenital intestinal anomalies can

be safely treated with resection and pri-mary anastomosis. Infants born with ilealor colonic atresia, however, generally havea profound disparity in diameters of theproximal and distal segments (Fig. 2B).While resection of part of the hugely di-lated proximal bowel and primary unionhas given good results in some cases, it isour belief that in most instances the Miku-licz resection and reconstruction is a more

sound approach. Indeed, in many cases thedisparity in bowel size makes the Mikulicz

procedure the only safe method of treat-ment. Not only is the strain on an anasto-mosis avoided, but the enterostomy allowsirrigation of the distal loop until a reason-

able size is achieved following which a

turn-in is readily accomplished.III. Intra-abdominal Catastrophes Re-

quiring Intestinal Resection. Many infantsand a certain number of children are seen

who have had some form of intra-peritonealdisaster. Newborn intestinal perforations,meconium peritonitis with obstruction, or

volvulus with gangrene are examples (Fig.2D). Even with successful management ofthe accompanying shock and sepsis, thesepatients demand the utmost skill and pre-

cise judgment on the part of the surgeon.The operation should be as short as possi-ble. Regional sepsis, intestine poorly suitedfor anastomotic suturing, and the precari-ous condition of the baby, make an exteri-orization procedure of the Mikulicz type

Volume 158Number 3

484clearly preferable. From our own experi-ence, we have become convinced that manyinfants and children with intra-abdominalcrises requiring intestinal resection havebeen saved by the Mikulicz procedure,when any other surgical plan would havebeen doomed to failure.

IV. Doubtful Viability of the Intestine.In the pediatric age group there are a num-

ber of conditions which lead to gangrene ofintestine; the most frequently encounteredone is malrotation with volvulus. Other less-common causes of infarction are congenitalbands, internal hernia, and postoperativeadhesions. In such patients, a large partor even all of the small intestine may beinvolved in a vascular compromise. A seg-

ment of the bowel may be clearly necrotic,yet a large part of the intestine may havequestionable viability. In such circum-stances, removal of all the discolored bowelwould result in an enteric cripple; some

more conservative measure is wiser. Ifonly the bowel which is obviously necroticis resected, and the questionably-viablepart can be given a chance to recover be-fore it is subjected to anastomosis or re-

ceiving a fecal stream, many gratifyingresults are obtained. By using the exteriori-zation and the double enterostomy, an anas-

tomosis is avoided in compromised boweland the intestinal ends may be observedso that their status can be continually eval-uated during subsequent hours or days.By this maneuver, the amazing ability ofintestine to recover from temporary vascu-

lar compromise allows the surgeon to sal-vage in a safe way, some intestine whichwould have been sacrificed if a primaryunion (requiring more extensive resectionto get back to healthy bowel) had beenattempted.

Operative Technic

It is difficult to improve on the concisedescription offered by Paul in 1895. In our

cases, the abdomen is usually exploredthrough a paramedian incision, but the

Annals of SurgerySeptember 1963

enterostomy can be brought out througha transverse incision or a rectus incision

with equal ease. As in any intestinal resec-

tion, the mesentery is first separated fromthe bowel which is to be removed. The le-sion is not resected at this stage, so theisolated segment appears as a bucket han-dle (Fig. 3A). Proximal and distal bowelare laid parallel in a double-barrel fashion,with the anti-mesenteric surfaces of thetwo loops in apposition to each other.These are then sutured together with two

rows of running fine silk, for a distance of5.0 to 7.0 cm., forming the spur. The buckethandle of diseased bowel is pulled out ofthe abdomen so that the double-barrelledintestine comes to lie appropriately at thelevel of the abdominal wall. The peri-toneum is closed with interrupted silk su-

tures and is anchored to the intestine whichprotrudes through it (Fig. 3B). Stitchesmust be carefully placed in the bowel wall,avoiding the mucosa to prevent the forma-tion of a fistula. The muscle fascia is closedwith a row of silk sutures and this layer isalso tacked to the bowel wall in severalplaces. The skin is then closed. In mostcases, it is best to stitch the skin edges tothe intestinal spur. Clamps are applied tothe two limbs, about 1.0 cm. above theskin level closure and the specimen is ex-

cised (Fig. 3C). A plastic surgical spray

can be applied to the wound and its suturesbefore transecting the intestine. The distalclamp can be allowed to remain in place,incorporating it in a dressing for one or

two days; this gives additional stabilizationto the spur while it is becoming sealed tothe abdominal wall. (During the exteriori-zation, we make no attempt to obliteratethe peritoneal space lateral to the spur.)

Management of Mikulicz Enterostomy

Following creation of the double-bar-relled enterostomy, several days are neces-

sary for healing of the surgical wound, and

stabilization of the patient. The often-

raised fear of fluid and electrolyte loss from

RANDOLPH, ZOLLINGER AND GROSS

Volume 158 MIKULICZ RESECTION INNumber 3

infant enterostomies is seldom seen. Ofnote, are the careful metabolic balancestudies of some of our patients by Richard-son,1 who showed that severe fluid loss israrely a problem, and even if it does occur,it can be successfully managed with carefulintake and output measurements and stand-ard intravenous replacement therapy.

q INFANTS AND CHILDREN 485

To cut down the common walls of thespur, a crushing clamp is usually appliedon the fourth or fifth postoperative day(Fig. 3E). If necessary, the clamp can beapplied even on the day of operation when-ever accelerated closure of the Mikulicz isdesirable (such as may be necessary if theenterostomy has been placed very high in

rC. -FIG. 3. Steps in performing a Mikulicz resection and establishment of a double-barrelled enteros-

tomy. A. Intestinal loop to be resected is freed from the mesentery. Anti-mesenteric surfaces of theintestine are joined with silk sutures (arrow). B. Peritoneum is closed with interrupted silk suturesand is sewn to the spur. C. Fascia and skin are closed, and the intestinal loop is resected. D. Twotypes of spur crushing clamps. E. Spur crushing clamp in place. F. Enterostomy dressing aroundclamp.

RANDOLPH, ZOLLINGER AND GROSS Annals of SurgerySeptember 1963

NONE liST.

INTERVAL'

FIG. 4. Graph showing follow up study in121 cases.

the ileum). The clamp usually cuts throughin four or five days. A gauze obstructivedressing is then applied over the stoma toencourage intestinal contents to run downinto the distal segment.

Enterostomy Closure

Closure of the enterostomy can be doneunder local or general anesthesia. The skinand fascial layers are carefully dissectedaway from the serosa of the stoma (whichis now a single opening). The previously-placed silk sutures serve as a guide, andshould be removed at the fascial level.When the sutures at the peritoneal levelare seen, the serosa of the stoma is carefullycleaned circumferentially and the stoma isinverted with two layers of interrupted silk.The closed bowel is now gently depressedand is covered by fascia of the abdominalmusculature which is approximated withinterrupted silk sutures. The skin is closedand the wound dressed.Of our 196 patients 147 came to closure.

Almost all of them had a standard extra-peritoneal closure. For nine it was neces-

sary to perform an intra-peritoneal proce-dure with revision and closure of the stoma

or removal of the spur with anastomosis ofthe intestinal limbs.

Analysis of Cases

Information acquired from the study of196 patients who had Mikulicz resectionsin the period 1940 through 1962 forms thebasis of this report. Long-term follow up

was possible in a large percentage of thepatients (Fig. 4). Most were seen for sev-

eral years, and a substantial number for as

long as 10 to 15 years after the surgery.

Thus, there is available a good samplingof both early and late complications aftera Mikulicz procedure. The cases were

grouped into six major categories, accord-ing to the principal diagnosis at the timeof operation.Meconium Ileus. There were 40 patients

treated by Mikulicz resection and subse-quent re-establishment of the intestinalcontinuity. Twenty-seven of these survivedand were cured of their obstruction. (Four-teen of these 27 who survived the abdomi-nal operation succumbed months or years

later from the pulmonary complications oftheir cystic fibrosis.) This record for reliefof meconium ileus obstruction is higherthan other methods reported in the litera-ture.

Intestinal Atresia. Thirty-nine babieswith intestinal atresia were treated byMukulicz exteriorization. Twenty-eight ofthese survived; a recovery of 71 per cent.For the ileal atresias, we have had a dis-tinctly higher rate of survivals followingexteriorization when compared to treatmentby primary anastomosis.

Intussusception. Today, with an in-creased awareness of the diagnosis of intus-susception, early treatment usually requiresonly simple reduction. A decade or twoago, late diagnosis was common, patientswere more often in shock, and antibioticswere not available; in desperately-ill pa-tients, some form of resection was fre-quently necessary. A Mikulicz resection

486

Volume 158 MIKULICZ RESECTION INNumber 3

proved to be highly efficacious in the group

of 30 patients, 26 of whom recovered.Neonatal Obstruction, with Perforation

or Peritonitis. Thirty-two infants had some

form of intra-peritoneal catastrophe withperforation and peritonitis. When resectionwas necessary in such patients, the Miku-licz procedure offered the least injuriousand quickest solution upon which we couldthen build further resuscitation of the pa-

tient. Seventeen, or more than half of thisgroup of critically-ill patients, survived.

Doubtful Viability of Bowel. The Miku-licz resection has been proven useful inpatients with marginal viability of a largeportion of intestine. Of 38 such babies,there have been 25 survivors. The 13deaths in this group were principally dueto irreparable vascular damage with mas-

sive loss of bowel.Miscellaneous Conditions. Among other

conditions treated by resection and double-barrelled enterostomy have been malignanttumors, mesenteric cysts, and intestinalduplications. Of 16 patients in the group,

12 are alive and well.

ComplicationsComplications Before Closure of En-

terostomy. Certain mechanical problemshave been encountered with the enteros-tomy before its closure (Table 2). Inade-quate cutting of the spur has been seen

most frequently, and is effectively treatedby re-application of the clamp for a fewdays. Skin breakdown has been amazinglyrare. Perforation of the intestine, assumedto be iatrogenic, has usually occurred dur-ing the irrigation of a distal loop. Thisproblem in seven patients emphasizes theneed for caution and gentleness in irriga-tion of any enterostomy. One patient withdehiscence of the wound had been closedwith catgut and serves to underscore our

preference for silk sutures.Forty-nine patients-about one-fourth of

the series-did not survive until closure ofthe enterostomy. Their disease process

INFANTS AND CHILDREN 487TABLE 1. Types of Patients Treated

uith Mikulicz Resection

I. Meconium ileus

II. Atresia or stenosis

Jejunal atresiaIleal atresiaIleal stenosisColonic atresiaColonic stenosis

III. Intussusception

IV. Intraperitoneal catastrophe

Meconium peritonitisNeonatal perforationPerforated viscus with peritonitisExstrophy of the cloaca

V. Marginal viability (including volvulus)

VI. Miscellaneous conditions

Omphalomesenteric ductMesenteric cystIntestinal duplicationMalignant tumorRevision of colostomy

Total

40

6225

42

33

161231

38

1

3543

196

TABLE 2. Complications in 196 PatientsBefore Closure of Ileostomy

Re-application of clamp necessary 13Mechanical problem with stoma

Retraction 6Stenosis 2Prolapse 1

Skin breakdown 4Perforation 7Abdominal abscess 3Dehiscence 1

Total 37

TABLE 3. Early Complications after147 Closures

Requiring NoOperation Operation

Partial obstruction 5Unrecognized distal obstruction 8Wound abscess I & D only 19Enterocutaneous fistula 5 24

Total 32 29

488 RANDOLPH, ZOLLINGER AND GROSS

TABLE 4. Late Complications*

Obstruction secondary to adhesions 13Obstruction at Mikulicz spur 10Jejuno-colic fistula 1Blind loop syndrome 4Incisional hernia 2

Total 30

* All of these patients are alive and well, except three.Of these three deaths: 1 died later of lymphosarcoma;1 died later of cystic fibrosis; 1 died of peritonitis.

would not tolerate even a Mikulicz resec-tion. Stated another way, if these childrencould not survive a Mikulicz procedure, thesimplest of all operations, they certainlywould not have lived after any other moreextensive procedure.

Early Complications Following StomalClosure. Following stomal closure, fivechildren showed evidence of partial intes-tinal obstruction; in each case this re-sponded to conservative therapy by gastricsuction (Table 3). After closure of thestoma, eight patients had evidence of ob-struction which was found to be caused bya separate congenital obstruction locateddistal to the turn-in. This blockage did not

a..

115

ISO

76

so

as

-ALIVE

ANLYSIOF

*DEATHS

I AEM? ISNMX

CYSTICe F0~

FIG. 5. Analysis of the mortality in 196 casesof Mikulicz resection.

Annals of SurgerySeptember 1963

become apparent until after the enteros-tomy was closed, when the bowel streamwas restored. Naturally, each of these re-quired secondary operation.Wound infection, including a small in-

cisional or stitch abscess, was fairly com-mon. Simple drainage generally handledthis effectively. However, some of these in-fections were forerunners of enteric fistu-lae. Of 29 such leaks, 24 spontaneouslyclosed.Late Complications. Obstruction sec-

ondary to peritoneal adhesions has occurredin 13 patients (Table 4). This number isnot surprising, since many of the subjectsoriginally had some degree of peritonitis.Ten children developed obstruction at thesite of the enterostomy, months or yearsafter it had been turned in. This complica-tion, which behaves clinically like an anas-tomotic stricture, requires operative relief,and is thought to represent incomplete cut-ting of the spur.

It is noteworthy that there have beenno instances of volvulus around the intra-abdominal portion of the Mikulicz spurwhich had been left attached to the ab-dominal wall. A jejuno-colic fistula, dis-covered nine years postoperatively, prob-ably occurred when the clamp extendedbeyond the depth of a short spur andcrushed an interposed knuckle of colon.Four patients with the blind loop syndromewere found; all responded well to simpleresection and intestinal anastomosis. Lastly,there were two incisional hernias.The important conclusion to be derived

from these observations is that 30 patientshave had late complications, yet all arenow alive except for three, of which onlyone had a cause of death (peritonitis) re-lated to the Mikulicz surgery (the othertwo being late fatalities from recurrentneoplasm or from cystic fibrosis).

MortalityOf the 196 patients studied, 121 are

alive and well, and 75 are dead.

0

gwlUz

i-L

Volume 158 MIKULICZ RESECTION IN INFANTS AND CHILDREN 489Number 3

Analysis of the deaths shows that 54 ofthe 75 patients died from causes unrelatedto their abdominal surgery (Fig. 5). Fortydied from the pulmonary complications ofcystic fibrosis (these included 27 patientswith meconium ileus, seven with meco-nium peritonitis, and six infants with in-testinal atresia). Other children who diedafter successful relief of their obstructionwere as follows: six from congenital heartdisease, four from omphaloceles, threefrom malignancy, and one from an acci-dental death.

In 21 patients who died there was nodisease other than their primary abdominalpathology. Thus, these deaths are clearlyrelated to the disease process or its surgicalcorrection. The diagnoses in these patientswere: intussusception-4; neonatal gastro-intestinal perforation-7; midgut volvuluswith marginal viability-5; intestinal atre-sia-4; and mesenteric cyst-1.

SummaryAlthough the trend among surgeons who

do significant numbers of pediatric ab-dominal cases is toward intestinal resec-tion with primary anastomosis, certain con-ditions are found in which a Mikuliczexcision constitutes a sounder choice. Theseinclude, meconium ileus, ileal or colonicatresia, intraperitoneal catastrophe, and ex-tensive intestinal vascular accident. Thepostoperative complications and their man-agement are apt to deter surgeons fromusing the Mikulicz resection more widely,but our experience leads us to believe thatthese are not too troublesome, are rarelyfatal, and certainly can be managed safely.It is our conclusion that the Mikulicz pro-

cedure offers the best salvage of manyseriously-ill patients. These convictions arebased on a series of 196 cases in infancyand childhood for whom a Mikulicz resec-tion was performed.

Bibliography1. Benson, C. D.: Resection and Primary Anas-

tomosis of the Jejunum and Ileum in theNewborn. Ann. Surg., 142:478, 1955.

2. Benson, C. D., J. P. Lloyd and J. D. Smith:Resection and Primary Anastomosis in theManagement of Stenosis and Atresia of theJejunum and Ileum. Pediatrics, 26:265,1960.

3. Bloch, O.: Quoted by von Mikulicz.84. Clatworthy, H. W. and J. R. Lloyd: Intestinal

Obstruction of Congenital Origin. Arch.Surg., 75:880, 1957.

5. Dennis, C.: Resection and Primary Anasto-mosis in the Treatment of Gangrenous orNon-reducible Intussusception in Children.Ann. Surg., 5:788, 1947.

6. Gerrish, E. W.: Operative Management ofCongenital Intestinal Atresia. Ann. Surg.,142:469, 1955.

7. Gross, R. E.: The Surgery of Infancy andChildhood. Philadelphia, W. B. SaundersCompany, 1953, 1,000 pp.

8. von Mikulicz, J.: Chirurgieische Erfahrungenuber das Darmcarcinoma. Archives Klin.Chir., 69:28, 1903.

9. Paul, F. T.: Colectomy. Liverpool Med. andChir. J., 15:374, 1895.

10. Rankin, F. W.: Resection and Obstruction ofthe Colon (obstructive resection). Surg.,Gynec. & Obst., 50:594, 1930.

11. Richardson, W. R.: Metabolic Studies on In-fants with Enterostomies. Surg. Forum, 7:75, 1957.

12. Swenson, O.: End-to-end Aseptic IntestinalAnastomoses. Surgery, 36:192, 1954.

13. Swenson, 0. and J. H. Fisher: Small BowelAtresia-Treatment by Resection and Pri-mary Aseptic Anastomosis. Surgery, 47:823,1960.

DiscussioN

DR. C. EVERETT KooP (Philadelphia): It is notunusual for someone from Bob Gross' service topresent a long and hazardous experience with sur-

gery and make it seem quite simple, and Dr.Randolph appears to be no exception to this.

The critics of the Mikulicz procedure are many,and I usually am among them if there is any